Transcript Rheumatoid Diseases

Musculoskeletal: Autoimmune;
Inflammatory; Metabolic; Infectious
Disorders
Rheumatoid Arthritis; SLE; Paget’s
Disease; Gout; Osteomyelitis
Autoimmune and Inflammatory
Disorders:
Rheumatoid Arthritis
Chronic systemic, inflammatory disease
characterized by recurrent inflammation of
connective tissue, primarily of synovial
joints and related structures.
Pathophysiology: Rheumatoid Arthritis
Cause unknown - Autoimmune Theory
• Exposure to unidentified antigen
• Triggers formation of an abnormal
immunoglobulin G (IgG)
• Autoantibodies called rheumatoid factor (RF)
develop in response to IgG
– ( Rheumatoid factor (RF) is diagnostic for RA)
• IgG + RF = IgG/RF called immune complexes
– Precipitates in synovial fluid & on articular
cartilage
– Triggers Inflammatory response
• Cartilage and connective tissue primarily
affected!
Pathophysiology: Rheumatoid Arthritis
Inflammatory response
• Immune complexes activate complement
• Neutrophils release proteolytic enzymes = damage or
thickening of synovial lining and damaged cartilage
• T helper CD4 cells stimulate release of cytokines such
as interleukin-1 and TNR (tumor necrosis factor alpha)
cause chondrocytes to attack cartilage. Primary drivers
of immune response in RA
• Chronic inflammation = hypertrophy of synovial
membranes, pannus formation, scarring, cartilage
destruction, disrupts tendons, ligaments
Joints changes
with RA
• Early Pannus
• Immune
complexes enter
joint
• Granulation,
inflammation at
synovial
membranes
• Cartilage softens
and begins
process of
destroying joint
RA
Mod advanced Pannus
joint cartilage disappears,
underlying bone destroyed,
joint surfaces collapse
Fibrous Ankylosis
Fibrous connective tissue replaces
pannus = loss of joint
motion
Bony Ankylosis
Eventual tissue and joint
calcification
Rheumatoid arthritis: assessment:
manifestations and complications
• Fatigue,
weakness, pain
• Joint deformity
• Rheumatic
nodules
• Multi-system
involvement
Joint Changes RA
• Bilateral &
symmetrical
• PIP’s (hands)
• MTP’s (feet)
• Thumb instability
• Swan neck
deformity
• Boutonniere
deformity
• Tensynovitis
• Subcutaneous
nodules
• Genu valgum
(knock-knee)
• Pes plano (flat foot)
• Gneu valgus
• Hallux valgus
• Prominent
metatarsal heads
• Hammer toes
Assessment RA
Deformities
that may
occur with
RA
Synotenovitis
Ulnar drift
Swan neck
deformity
Boutonniere
deformity
Mutlans deformity
(rapidly progressing
RA)
Hitch-hiker thumb
Genu valgus
Hallux valgus
Hammer toes
Subcutaneous
nodules (disappear
and appear without
warning)
Manifestations of RA
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Systemically ill
Hematologic
Pulmonary/CV
Neurologic
Ocular symptoms
(Sjogren’s & Felty)
• Skin
• Musculoskeletal
deformity, pain
Pain!
Pain!
Pain!
Extra-articular Manifestations
• Can affect almost all systems
• Sjogren’s syndrome: 10-15% of
patients. Decreased lacrimal and
salivary gland secretions
• Felty Syndrome: most common in
pts with nodule forming type Inflammatory eye disorders,
splenomegaly, lymphadenopathy,
pulmonary disease, blood
dyscrasias
Diagnostic Tests RA
+ RF
RF titer – 80%
ESR elevated
C-reactive protein – general
indicators of inflammation
CBC
Synovial fluid – WBC’s present
bone scans – early detection
Swelling,inflammation
X-rays only useful in late stages
How does Rheumatoid Arthritis Compare
to Osteoarthritis?
• Definition: *wear and tear,
progressive, non-systemic,
Degenerative Joint Disease (DJD)
• Pathophysiology – loss of cartilage,
exposed bone, bone spurs,
inflammation
Comparison of RA and OA
RA
Cause unknown; auto-immune factor, genetic?
Onset sudden with Remissions
*Body parts affected: systemic, small joints, symmetrical
Causes redness, warmth, swelling of joints
Females, begins at any age; 2-3:1 ratio
RF is positive, pain increases with movement
OA
Cause “wear and tear”, develops slowly
Non-systemic, weight bearing joints
Middle-aged, elderly, males 2-1 affected, Begins after 40
Does not cause malaise
RF is negative, pain decreases with movement
Osteoarthritis (top
slide only)
Identify which joints
are primarily
affected with
osteoarthritis.
What factors
contribute to the
development of
osteoarthritis?
Structural changes with Osteoarthritis
Early
Cartilage softens, pits, frays
Progressive
Cartilage thinner, bone ends hypertrophy,
bone spurs develop and fissures form
Advanced
Secondary inflammation of synovial
membrane; tissue and cartilage destruction; late
ankylosis
What signs and
symptoms does
the person with
osteoarthritis
experience?
Normal Knee
structure
Moderately
advanced
osteoarthritis
Advanced
osteoarthritis
What symptoms/assessment for the
patient with osteoarthritis?
Onset of pain is insidious, individual is
healthy!
Pain is aching in nature; relieved by rest!.
Local signs and symptoms: swelling,
crepitation of joint and joint instability,
asymmetrical joint involvement
Deformities with Osteoarthritis
Carpometacarpocarpal joint of
thumb with subluxation of the
first MCP
Genu varus
Herberden’s nodes
Osteoarthritis (review
• Nursing diagnosis
only)
• Diagnostic Tests
– None specific
– Late joint
changes, boney
sclerosis, spur
formation
– Synovial fluid
inc., minimal
inflammation
– Gait analysis
• Interventions
determined by
complications
– Supportive devices
– Medications (no
systemic treatment
with steroids)
– Dietary to dec. wt.
– Surgical
Intervention (joint
replacement)
– Teaching
Interventions RA
• Nursing
Diagnosis
– Comfort
– Physical mobility
– Self image
• Goals
• Team Approach
• Pain management
• Exercise
• Surgery
– Synovectomy
– Joint fusion
• Athrodesis
– Joint replacement
• Arthroplasty
• Teaching
Medications RA
• ASA & NSAIDS, Cox-2 inhibitors
• Corticosteroids; low dose
• DMARDs (diverse group) of remitting
agents: including antimalarial
(hydroxychloroquine: plaquenil) *loss of vision
Penicillamine (empty stomach); bone marrow/kidney issues
Gold (Auranofin) *dermatitis, blood dyscrasias, renal
toxicity
• Immunosuppressive agents as methotrexate
and cyclosporine
• Biologic response modifiers
– Adalimumab (Humira)- Sub-Q binds with tumor necrosis factor to
decrease inflammatory process – report infections STAT
– Infliximab (Remicade)- IV similar to above
Joint Protection: Do’s and Don’t’s
Case Presentation; Mrs. Michaels with
Rheumatoid Arthritis
(PDS: Adult Health: Musculoskeletal Health: Mrs. Michaels)
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Comparison to ‘usual’ course
Diagnostic tests
Nursing diagnosis
Therapies
– Medications used
– Exercise
– Joint Protection
• Resources on the Web
Systemic Lupus Erythematous
(SLE)
Chronic multisystem disease
involving vascular and
connective tissue
Lupus
Foundation
• Exact etiology unknown:
Etiology and
genes (HLA), hormones,
Pathophysiology SLE
environment involved
• Formation auto-antibodies
• Course of disease
to DNA; immune
varies
complexes deposited
– Mild
– Episodic
• Inflammatory response
– Rapidly fatal
triggered by deposition of
immune complexes in skin
kidney, heart, joints, brain,
lung, spleen, GI
• Drug induced syndrome
similar to SLE (Procan-SR,
Pronestal, (Apresoline)
hydralazine, isonaiazid,
siezure meds)
Manifestations and Complications
• Dermatologic – 50% have butterfly rash. Skin,
nasal, oral lesions, hair loss.
• Musculoskeletal – polyarthralgia, arthritis,
swelling, pain, deformity
• Cardiopulmonary – Tachypnea, cough, fibrosis
of nodes = arrhythmias, accelerates CAD
• Renal – 50% of patients within 1 year of onset.
Protienurea to rapid glomerulonephritis
• CNS – focal seizures, peripheral neuropathy,
organic brain syndrome
• Hematologic – antibodies form against blood
cells
• Infection – major cause of death
Manifestations/Complications
of SLE
Incidence: 1:2000
• Women 9:1, childbearing age, african
american, native
american, asian
• Periods remission
and exacerbation
• Stress
• Environmental
factors
• Assessment
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Low grade fever
Integumentary
MS involvement
CV
Respiratory
Urinary Renal failure
Neurologic CNS
GI
Hematologic
Endocrine
Reproductive
Characteristic
butterfly rash
associated with
SLE, especially
discoid lupus
erythematous
SLE characterized by periods of
remission and exacerbation.
Stimulated by sunlight, stress,
pregnancy, infections like strep and
some drugs.
Some drugs like apresoline,
pronestyl, dilantin, tetracycline,
phenobarbital may cause a lupus-like
reaction which disappears when drug
is stopped.
Diagnostic Tests
• LE cell prep; + in
other rheumatoid
diseases
• Anti-DNA- specific
• Anti nuclear
antibody, titer
• Serum Complement
levels decreased
• ESR - elevated
• CBC- leuko /lympho
cytopenia, anemia
• UA –RBC’s, protien
• Kidney biopsy
• Criteria to Dx.
– malar, discoid
rash; oral ulcers
– photosensitivity
– arthritis
– renal disorder
– immunological
disorder
– DNA:ANA
Therapeutic
Interventions/Management SLE
• Medications
– NSAIDS
– (Disease modifying agents)
Antimalarial drugs
– Corticosteroids
– Immunsuppressive therapy
• Antineoplastic drugs
such as Imuran, cytoxan,
cyclosporine
• Avoid UV light
• Reduce stress
• Monitor/manage to prevent
complications
• Nursing diagnosis
– See RA
• Impaired skin integrity
• Ineffective protection
• Impaired health
maintenance
• Goal: control
inflammation
• Emotional support
• Life Planning
•
Required Review
Case Study
•
Clinical Background:
•
18 year old patient admitted with
recent onset (3 months earlier) of
malar rash and constitutional
symptoms (weakness and malaise),
now symptoms of renal failure. She
reported having a 5-year history of
Raynaud's phenomenon and
arthralgia.
•
Abnormal results of laboratory
studies included the presence of ANA,
anti-nDNA, anti-SS-A autoantibodies
and a proteinuria of approximately 10
g/d.
Renal biopsy revealed a Class IV
lupus glomerulonephritis.
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What assessment data is priority:
what additional date should you
collect?
What are the priority nursing
problems?
What are the priority
interventions?
What medications are
typically used and why?
Scleroderma
(Systemic sclerosis)
• Definition: progressive sclerosis of
skin and connective tissue; fibrous and
vascular changes in skin, blood
vessels, muscles, synovium, internal
organs. become “hide bound”
• Immune-mediated disorder; genetic
component
Scleroderma (Systemic sclerosis)
• Abnormal amounts of fibrous
connective tissue deposited in skin,
blood vessels, lungs, kidneys, other
organs
• Can be systemic or localized (CREST)
syndrome
CREST Syndrome
• Calcinosis
• Raynaud’s
phenomena
• Esophageal
hypomotility
• Sclerodactyl (skin
changes of
fingers)
• Telangiectasia
(macula-like
angioma of skin)
Crest Syndrome
More on
CREST
CREST Syndrome & scleroderma
Sclerodactyly
(localized scleroderma
of fingers)
Raynaud’s disease with
ischemia
Typical “hidebound” face of
person with
scleroderma
Tissue hardens;
claw-like
fingers; fibrosis
Scleroderma Manifestations &
Complications (systemic)
• Female 4:1
• Pain, stiffness,
polyartheritis
• Nausea, vomiting
• Cough
• Hypertension
• Raynauld’s
syndrome
• Skin atrophy,
hyperpigmented
Scleroderma cont.
• Esophageal
hypomotility leads to
frequent reflux
• GI complaints
common
• Lung-pleural
thickening and
pulmonary fibrosis
• Renal
disease...leading
cause of death!
Diagnosis/Treatment
Scleroderma
• R/O autoimmune disease •
• Radiological: pulmonary
fibrosis, bone resorption,
subcutaneous calcification,
distal esophageal
hypomotility
• ESR elevated
• CBC anemia
• Gammaglobulin levels
elevated;
• RF in 30%& SCL-70 in 35%
• Skin biopsy to confirm
What are the KEY
components of care for
the individual with
Scleroderma?
Scleroderma: Patient Care
• Do’s
– Avoid cold
– Provide small,
frequent feedings
– Protect fingers
– Sit upright post
meals
– No fingersticks
– Daily oral hygiene
Resources
Scleroderma: Patient Care
• Medications: based upon
symptoms:
– Immunosuppressive
agents & steroids &
remitting agents
– Ca channels blockers &
alpha-adrenergic
blockers
– H2 receptor blockers
– ACE inhibitors
– Broad spectrum
antibiotics
– Capsaicin
Ankylosing Spondylitis
• Definitions: chronic
inflammatory
polyarthritis of spine
• Affects mostly young
men
• Associated with HLAB27 antigen
– positive antigen (90%)
• Pathophysiology
& Manifestations
– Like arthritis have
inflammatory changes;
erosion of cartilage,
ossification of joint
margins; scar tissue
replaces
– Morning backache, flexion
of spine, decreased chest
expansion
• Diagnosis
– ESR elevation
– Positive HLA-B27
antigen
– Vertebral changes
Ankylosing
Spondylitis
Insidious
onset
Morning
backache
Inflammation
of spine; later
spine
ossification
Oh my back hurts!
Ankylosing
spondylitis
Identify a
PRIORITY
nursing
concern
related to
ankylosing
spondylitis
Management Ankylosing Spondilitis
• Do’s
– Maintain spine
mobility
– Pain
management
– Proper
positioning
– Meds for pain,
inflammation
Other Collagen Diseases
• Lyme Disease
(mimics rheumatoid
disease)
– Caused by spirochete,
borrelia burgdorferi (tick)
– Inflammatory disorder
– 3 stages
• Initial rash (target)
• Disseminated (arthritic
like symptoms)
• Late (neurologic
symptoms)
• Diagnosis
– Culture (difficult)
– Antibody detection
Treatment: antibiotics
(amoxicillin, vibramycin,
tetracycline, etc);
NSAID)
Prevention
Other Collagen Diseases
• Polymyositis
– Systemic connective
tissue disorder
characterized by
inflammation of
connective tissue and
muscle fibers
– Autoimmune;
affecting women 2:1
– If muscle fiber
inflammation is
accompanied by skin
lesion disease known
as dermatomyositis
• Manifestations &
complications
– Muscle pain, tenderness,
rash; arthralgias; fatigue;
fever and weight loss;
– Skeletal muscle weakness
most prominent
– Dusky red rash over face
– Raynaud’s phenomenon
– Malignancy with
dermatomyositis
• Diagnosis
– None specific
– Elevated CK
Other Collagen Diseases
cont
• Reiters
syndrome
– Self-limited disease of
reactive arthritis
• Adult males infection
of eyes and urethra
• infections such as:
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Chlamydia
Shigella
Salmonella
Enterocolitis
• associated with HLA—
B27 antigen
Antibiotics &
pain meds
Joints of
ankles, feet.
& sacroiliac
Persists and
returns
Other Collagen Diseases
cont.
–Polyarteritis
Nodosa
• Collagen;
• diffuse inflammation
and necrosis of wall of
small to medium sized
arteries
• Especially in
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Muscles
Kidneys
heart
liver,
GI and peripheral
nerves like SLE
– Juvenile
Rheumatoid
Arthritis